{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,27]],"date-time":"2026-01-27T09:55:57Z","timestamp":1769507757883,"version":"3.49.0"},"reference-count":52,"publisher":"Georg Thieme Verlag KG","issue":"23","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dtsch Med Wochenschr"],"published-print":{"date-parts":[[2024,11]]},"abstract":"<jats:p>Vor 100 Jahren hat Dr. Eli Moschcowitz den ersten Fall einer thrombotisch-thrombozytopenischen Purpura beschrieben. Viele Jahrzehnte gab es keine anerkannten Therapieoptionen und die Letalit\u00e4t war extrem hoch. Anfang der 90iger-Jahre setzte sich dann zunehmend die Therapie mit Steroiden und Plasmaaustausch durch, wobei die Letalit\u00e4t immer noch \u00fcber 20\u200a% lag. Es dauerte bis kurz vor die Jahrtausendwende, bis in Bern und New York die Krankheitsmechanismen (ADAMTS13-Defizienz) entschl\u00fcsselt und somit der Weg f\u00fcr neue Therapieoptionen frei gemacht wurde. Es wurde nun klar, dass die erworbene TTP (iTTP) eine Autoimmunerkrankung ist und der Autoantik\u00f6rper gegen ADAMTS13 gerichtet ist, eine Protease, die gro\u00dfe von-Willebrand Faktor-Multimere fragmentiert. Dadurch entsteht ein schwerer ADAMTS13-Mangel. Die Multimere \u00fcberschie\u00dfender Gr\u00f6\u00dfe persistieren und binden Thrombozyten, wodurch mikrovaskul\u00e4re Thrombosierungen entstehen. Abgegrenzt davon wird die kongenitale TTP (cTTP), die durch Mutationen im ADAMTS13-Gen verursacht wird (Upshaw-Schulman-Syndrom). Bei anderen Formen der thrombotischen Mikroangiopathie (TMA, z.\u200aB. aHUS) tritt keine schwere ADAMTS13-Defizienz auf. Zwei randomisierte kontrollierte Studien belegten den Nutzen des 2019 zugelassenen, selektiven bivalenten Anti-von-Willebrand-Faktor (vWF) Nanobodies Caplacizumab in der Behandlung der iTTP. Diverse Publikationen nationaler iTTP-Kohorten verbesserten die Datenlage und zeigten konsistente Verringerungen der Zeit bis zur Normalisierung der Thrombozyten, eine drastische Reduktion refrakt\u00e4rer Verl\u00e4ufe und Exazerbationen (insbesondere bei Therapiesteuerung nach ADAMTS13-Aktivit\u00e4t) sowie Hinweise f\u00fcr eine verringerte Mortalit\u00e4t, die insbesondere bei korrekter und fr\u00fchzeitiger Anwendung der heutigen Therapieoptionen (Plasmaaustausch, Steroide, Caplacizumab, Rituximab) bei 2\u20136\u200a% anzusetzen ist. Moderne Therapieoptionen umfassen Strategien zur pr\u00e4emptiven Therapie bei ADAMTS13-Rezidiven sowie die plasmaaustauschfreie Behandlung. M\u00f6glicherweise wird zuk\u00fcnftig der Einsatz von rekombinantem ADAMTS13, auch bei iTTP-Patienten, die therapeutischen Optionen erweitern.<\/jats:p>","DOI":"10.1055\/a-2360-8725","type":"journal-article","created":{"date-parts":[[2024,11,6]],"date-time":"2024-11-06T23:39:44Z","timestamp":1730936384000},"page":"1423-1430","source":"Crossref","is-referenced-by-count":2,"title":["100 Jahre Thrombotisch-thrombozytopenische Purpura (TTP) \u2013 Was\u00a0haben wir gelernt?"],"prefix":"10.1055","volume":"149","author":[{"given":"Ralph","family":"Wendt","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Nephrologie, Klinikum St. Georg, Leipzig, Delitzscher Str. 141, 04129 Leipzig, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Linus","family":"V\u00f6lker","sequence":"additional","affiliation":[{"name":"Klinik II f\u00fcr Innere Medizin und Zentrum f\u00fcr Molekulare Medizin K\u00f6ln (ZMMK), Fakult\u00e4t f\u00fcr Medizin, Universit\u00e4t zu\u00a0K\u00f6ln, Uniklinik K\u00f6ln, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin","family":"Bommer","sequence":"additional","affiliation":[{"name":"Alb-Fils-Kliniken G\u00f6ppingen, Klinik f\u00fcr H\u00e4matologie, Onkologie, Infektiologie und Palliativmedizin, Eichertstra\u00dfe 3, 73035 G\u00f6ppingen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marc","family":"Wolf","sequence":"additional","affiliation":[{"name":"Neurologische Klinik, Katharinenhospital, Klinikum Stuttgart, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Charis","family":"von Auer","sequence":"additional","affiliation":[{"name":"III. Medizinische Klinik und Poliklinik f\u00fcr H\u00e4matologie und Medizinische Onkologie, Universit\u00e4tsmedizin der Johannes-Gutenberg-Universit\u00e4t Mainz, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lucas","family":"K\u00fchne","sequence":"additional","affiliation":[{"name":"Klinik II f\u00fcr Innere Medizin und Zentrum f\u00fcr Molekulare Medizin K\u00f6ln (ZMMK), Fakult\u00e4t f\u00fcr Medizin, Universit\u00e4t zu\u00a0K\u00f6ln, Uniklinik K\u00f6ln, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul","family":"Brinkk\u00f6tter","sequence":"additional","affiliation":[{"name":"Klinik II f\u00fcr Innere Medizin und Zentrum f\u00fcr Molekulare Medizin K\u00f6ln (ZMMK), Fakult\u00e4t f\u00fcr Medizin, Universit\u00e4t zu\u00a0K\u00f6ln, Uniklinik K\u00f6ln, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wolfgang","family":"Miesbach","sequence":"additional","affiliation":[{"name":"Schwerpunkt H\u00e4mostaseologie\/H\u00e4mophiliezentrum, Medizinische Klinik II, Universit\u00e4tsklinikum Frankfurt, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul","family":"Kn\u00f6bl","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Innere Medizin I \u2013 Abteilung f\u00fcr H\u00e4matologie und H\u00e4mostaseologie, Medizinische Universit\u00e4t Wien, \u00d6sterreich"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2024,11,6]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"567","DOI":"10.1016\/0002-9343(52)90022-3","article-title":"An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries An undescribed disease","volume":"13","author":"E 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